Exploring perivascular epithelioid cell tumors (PEComas) in the CNS: insights from two case reports and a comprehensive literature review.

Perivascular epithelioid cell tumors (PEComas) represent a rare and intriguing subset of neoplasms within the central nervous system (CNS). This report describes two unique cases that highlight distinct origins and clinical behaviors, offering valuable contributions to the limited body of knowledge regarding CNS PEComas. Despite their rarity, PEComas should be considered in the differential diagnosis of mesenchymal tumors that display an atypical combination of clinical, radiological, and histomolecular features, especially when melanocytic and/or myoid markers are present. Treatment with mTOR inhibitors can potentially stabilize the disease in a significant number of more aggressive PEComas, even those located in the CNS.